The arteriovenous malformation of the vein of Galen (AVG), composed of a fistula between medium to large arteries and the vein of Galen, is frequently fetal in the neonatal period. We now report three cases of infants with AVG and associated patent ductus arteriosus (PDA), and review the literature for these associated findings. The presence of a PDA can result in severe vascular derangement in the postoperative period, and further complicate management of these infants. Analysis of this abnormal central circulation may contribute to a better understanding and therefore outcome for this unfortunate group of patients. © 1990 Springer-Verlag.